Q21
Conditions
Interventions
Group 1: 15 severly symptomatic patients with congenital heart disease (max. O2 consumption of less than 20 ml/kg/min and NYHA functional class II-III) will undergo a comprehensive and unique set of l
Sponsors
Institut für Schlafmedizin und neuromuskuläre Erkrankungen Universitätsklinikum Münster
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: a. Age > 18 years (no age limit) (Group 1, 2 and 3) b. able to consent (Group 1, 2 and 3) c. Diagnosis of congenital heart disease according to the most recent Guidelines of the European Society of Cardiology (ESC); >12 weeks since the diagnosis of congenital heart disease has been made; no hospitalisation for HF in the last 4 weeks; optimal medical therapy in accordance with the most recent Guidelines of the European Society of Cardiology (ESC) with no change in medication in the last 4 weeks and combination therapy for pulmonary arterial Hypertension due to congenital heart disease; where there was no treatment with combination therapy in PAH patients then the reason must be documented d. Controls (matched for age and gender) with normal findings on echocardiogram, electrocardiogram and normal levels of natriuretic peptide hormone
Exclusion criteria
Exclusion criteria: a. Body Mass Index (BMI) > 35 b. Chronic obstructive pulmonary disease, emphysema c. Presence of severe neurological comorbidities especially primary myopathy, muscular dystrophy, motor neuron disease, phrenic nerve disease, epilepsy, multiple sclerosis, parkinson’s disease d.Severe psychiatric disease including dementia e. Cardiac pacemaker
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Twitch transdiaphragmatic pressure after magnetic cervical stimulation of the phrenic nerves [cmH2O] [after Placement of two thin balloon catheters above and below the diaphragm allowing for continuous measurement of transdiaphragmatic pressure] | — |
Secondary
| Measure | Time frame |
|---|---|
| Fatigue Severity Scale [points], SF-36 [points], 6 MWD [m], Medical Research Council Dyspnoea Scale [points], Modified Borg Dyspnoea Scale [points], ->the above mentioned Parameters will be assessed by means of questionaires peak V O2 oxygen uptake [ml O2/min/kg], fist muscle strength [kg], leg muscle strength [kg], ->the above mentioned Parameters will be assessed by means of dynamometers Forced vital capacity [ml], FEV1/FVC [%], MIP [cmH2O], MEP [cmH2O], SNIP [cmH2O], peak cough Flow [L/sek], ->the above mentioned Parameters will be assessed by means of handheld spirometry devices Rate of rise of diaphramatic amplitude during tidal breathing on ultrasound [cm/sec], Rate of rise of diaphramatic amplitude during Sniff maneuver on ultrasound, Rate of rise of diaphramatic amplitude during maximal inspiration on ultrasound, diaphragm thickness after maximal inspiration on ultrasound [cm], diaphragm thickness after maximal exspiration on ultrasound, diaphragm thickness after tidal breathing on ultrasound [cm], thickening ratio [n.u.], ->the above mentioned Parameters will be assessed by means of diaphragm ultrasound twitch mouth pressure after magnetic cervical stimulation of the phrenic nerves [cmH2O], twitch mouth pressure after cortical stimulation of the phrenic nerves [cmH2O], twitch transdiaphragmatic pressure after magnetic cortical stimulation of the phrenic nerves [cmH2O], twitch gastric pressure after thoracic magnetic stimulation of the (expiratory) abdominal muscles [cmH2O] twitch mouth pressure after thoracic magnetic stimulation of the (expiratory) abdominal muscles [cmH2O] phrenic nerve conduction time after cortical magentic stimulation [ms], phrenic nerve conduction time after cervical magentic stimulation [ms], Amplitude of compound diaphragm muscle action potential after cortical magentic stimulation [mA], Amplitude of compound diaphragm muscle action potential after cervical magnetic stimulation [mA], central motor conduction time [ms], transdi | — |
Countries
Germany
Contacts
Public ContactPaul-Gerhard Diller
Klinik für Kardiologie III Universitätsklinikum Münster
Outcome results
None listed